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镰状细胞特征和α-地中海贫血症中骨骼肌微血管的重塑。

Remodeling of skeletal muscle microvasculature in sickle cell trait and alpha-thalassemia.

机构信息

Laboratoire de Physiologie de l'Exercice, Université de Savoie, Chambéry, France.

出版信息

Am J Physiol Heart Circ Physiol. 2010 Feb;298(2):H375-84. doi: 10.1152/ajpheart.00812.2009. Epub 2009 Nov 13.

DOI:10.1152/ajpheart.00812.2009
PMID:19915173
Abstract

The influence of sickle cell trait and/or alpha-thalassemia on skeletal muscle microvascular network characteristics was assessed and compared with control subjects [hemoglobin (Hb) AA] in 30 Cameroonian residents [10 HbAA, 5 HbAA alpha-thalassemia (alpha-t), 6 HbAS, and 9 HbASalpha-t] matched for maximal work capacity and daily energy expenditure. Subjects performed an incremental exercise to exhaustion and underwent a muscle biopsy. Muscle fiber type and surface area were not different among groups. However, sickle cell trait (SCT) was associated with lower capillary density (P < 0.05), lower capillary tortuosity (P < 0.001), and enlarged microvessels (P < 0.01). SCT carriers had reduced counts of microvessels <5-microm diameter, but a higher percentage of broader microvessels, i.e., diameter >10 microm (P < 0.05). alpha-Thalassemia seemed to be characterized by a higher capillary tortuosity and unchanged capillary density and diameter. Thus, while SCT is a priori clinically benign, we demonstrate for the first time that significant remodeling of the microvasculature occurs in SCT carriers. These modifications may possibly reflect protective adaptations against hemorheological and microcirculatory dysfunction induced by the presence of HbS. The remodeling of the microvascular network occurs to a lesser extent in alpha-thalassemia. In alpha-thalassemic subjects, increased capillary tortuosity would promote oxygen supply to muscle tissues and might compensate for the lower Hb content often reported in those subjects.

摘要

研究人员评估了镰状细胞特征和/或α-地中海贫血对骨骼肌微血管网络特征的影响,并与 30 名喀麦隆居民(10 名血红蛋白(Hb)AA、5 名 HbAAα-地中海贫血(α-t)、6 名 HbAS 和 9 名 HbASα-t)进行了比较,这些居民的最大工作能力和日常能量消耗相匹配。受试者进行了递增至力竭的运动,并进行了肌肉活检。各组之间的肌纤维类型和表面积没有差异。然而,镰状细胞特征(SCT)与较低的毛细血管密度(P < 0.05)、较低的毛细血管扭曲度(P < 0.001)和较大的微血管(P < 0.01)相关。SCT 携带者的微血管计数<5-μm 直径减小,但较宽的微血管,即直径>10μm 的百分比增加(P < 0.05)。α-地中海贫血似乎表现为较高的毛细血管扭曲度和不变的毛细血管密度和直径。因此,虽然 SCT 临床上是良性的,但我们首次证明,SCT 携带者的微血管发生了显著的重塑。这些改变可能反映了对由 HbS 引起的血液流变学和微循环功能障碍的保护适应。α-地中海贫血患者的微血管网络重塑程度较小。在α-地中海贫血患者中,毛细血管扭曲度的增加将促进向肌肉组织供氧,并可能补偿这些患者中经常报道的 Hb 含量较低的情况。

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